Malignant histiocytosis X. A distinct clinicopathologic entity

Abstract

Histiocytosis X (HX) is characterized morphologically by a proliferation of Langerhans' cells (LC), and most often has an indolent, chronic course. To determine whether a distinct clinicopathologic entity of malignant histiocytosis X exists, the authors examined tissues from 31 patients with HX and divided them into four categories. Group A (19 patients) was characterized morphologically by benign‐appearing LC and had an indolent course. The male:female (M:F) ratio was 10:9, and the mean age was 21 years (range, 2 months to 60 years). The immunophenotype of this group was S‐100+, vimentin+, LN‐2+, LN‐3+, lysozyme−, LCA−, Leu‐M1−. Group B (three patients) had benign‐appearing LC, yet had an aggressive clinical course. All patients were male, with a mean age of 47 years (range, 3 years to 72 years). Organs involved included the liver, spleen, heart, thymus, lung, kidney, and pancreas. The immunophenotype was the same as for Group A. Group C (two patients) had atypical and malignant appearing LC, yet a relatively benign clinical course. The ages were four and 65 years, with one female and one male patient. In both patients, the cells were S‐100+, vimentin+, LN‐2+, LN‐3+, and LCA−. Group D (seven patients) was characterized by atypical and malignant‐appearing LC and an aggressive clinical course. The mean age was 25 years (range, congenital to 54 years) with one female and six male patients. Organs involved were the thymus, lungs, spleen, liver, kidney, brain, heart, pancreas, stomach, and muscle. Birbeck granules were found in two patients, and the one patient on which fresh tissue was available was CD1+. The typical immunophenotype was S‐100+, vimentin+, LN‐2+, LN‐3+, Leu‐M1−, lysozyme—. The results of our study indicate that (1) a distinct clinical entity of malignant HX, characterized morphologically by malignant‐appearing LC and clinically by male predominance, atypical organ involvement, and an aggressive clinical course, does exist; and (2) the morphologic appearance of the LC is an imperfect predictor of the clinical severity of HX.

Langerhans cell sarcoma: a case report and review of the literature

“…Other tumours, such as metastatic melanoma and metastatic undifferentiated large-cell carcinoma, have also been confused with LCS [37,39].…”

“…Ben-Ezra et al, however, suggest that in the case of LCH, morphology is only an imperfect predicator of the clinical outcome. They described cases of benign cytological findings and aggressive clinical course and cases of malignant cytological findings and benign clinical outcome [37].…”

“…Inflammatory cell infiltration, consisting of lymphocytes and eosinophil gran- Table I. Summary of the reported cases of Langerhans cell sarcoma [2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35] ulocytes, was also found, and isolated mitotic figures were identified ( Fig. 3A and 3B).…”

Cutaneous Langerhans Cell Sarcoma: A Case Report

“…They can occur at any age, although incidence peaks around the fourth decade of life. Skin and the underlying soft tissues are the most common sites of tumor origin, but the involvement of bone, lung, gallbladder, and peritoneal lymph nodes has also been described [5][6][7]1].…”

Fine Needle Aspiration Biopsy Diagnosis of Histiocytosis-X: A Brief Review

“…Immunocytochemistry may also help to confirm the diagnosis of Hx by precise identification of LC. Several studies have helped to delineate the immunohistochemical staining patterns of LC [21][22][23]. Thus, LC are generally negative for leukocyte common antigen, nonspecific esterase, Leu Ml, epithelial membrane antigen (EMA) and LN1.…”